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Macmillan and Cancerbackup merged in 2008. Together we provide free, high quality information for people affected by cancer through our publications, website and phone service. Find out more| .
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This information is about a condition called Paget’s disease of the breast. It should ideally be read with our general information about breast cancer| and ductal carcinoma in situ (DCIS)| .
Paget’s disease of the breast is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have it have an underlying breast cancer. The underlying breast cancer may be an invasive breast cancer or ductal carcinoma in situ (DCIS). In DCIS, the cancer cells are completely contained within the milk ducts.
Paget’s disease affects about 1–2 out of every 100 women with breast cancer. It is most common in women in their 50s, but can occur at a younger or older age. It can affect men, but this is extremely rare.
The cause of Paget's disease is unknown, but certain women seem to be at a higher risk of developing breast cancer. This includes women who have never had children or had them late in life, women who started their periods at a young age or who had a late menopause, and women who have a strong family history| of breast cancer.
The first symptom is usually a scaly, red rash affecting the nipple and sometimes the dark area of skin surrounding the nipple (the areola). The rash always affects the nipple first, and may then affect the areola. It doesn't go away and may become sore.
The skin of the nipple and areola may be inflamed. There may also be crusting, bleeding and ulceration. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area of cells. The nipple may turn inwards (be inverted). There may or may not be a lump in the breast.
Around half of women who have Paget's disease have a breast lump that can be felt at the time it is diagnosed.
Paget’s disease can be confused with other skin conditions such as eczema, dermatitis or psoriasis, as they can look very similar. This can make Paget’s disease difficult to diagnose.
Paget’s disease usually affects the nipple first and then the surrounding tissue. Other skin conditions usually affect the areola (the dark area of skin around the nipple) first, and then spread into the nipple.
Several tests may be carried out to diagnose Paget’s disease of the breast. They may include the following:
Mammograms| may be used to look for changes in the affected breast, and to check the other breast.
A gel is spread on the breast and a small device, which emits sound waves, is rubbed over the area. The echoes are converted into a picture of the breast tissue by a computer. This test is painless and takes just a few minutes.
This is the main test for cancer cells below the skin surface. A small sample of skin and underlying breast tissue is taken and sent to the laboratory to be examined under a microscope. A local anaesthetic is usually given before the biopsy is carried out, to numb the area first. The biopsy may be taken at the same time as an ultrasound, to ensure that it is taken from the abnormal area.
Cells from the affected area can be scraped, or pressed, onto a glass slide to be examined under a microscope.
The treatment of Paget's disease of the breast will depend on:
Surgery| is the main treatment for Paget’s disease. This may involve an operation to remove all of the breast (a mastectomy) or an operation to remove the affected area of breast, including the nipple and areola, and some surrounding normal breast tissue (breast-conserving surgery). Some of the lymph nodes in the underarm may also be removed.
A mastectomy may be recommended if the cancer is affecting a wide area, is close to the nipple, or if there is DCIS in a number of areas in the breast (multifocal). It may be possible to have breast reconstruction at the same time as a mastectomy or as a second operation some months later.
Breast‑conserving surgery may be possible if the cancer, or DCIS, is close to the nipple and only affecting a small area of breast tissue.
Some people don't need any further treatment after surgery. Others may have radiotherapy, hormonal therapy or chemotherapy. These treatments may be given separately or together.
Radiotherapy| treats cancer by using high‑energy x-rays to destroy the cancer cells, while doing as little harm as possible to the healthy cells. Radiotherapy to the remaining breast tissue is usually recommended after breast-conserving surgery.
Hormonal therapies| are commonly used for treating breast cancer. They reduce the production of hormones within the body, or prevent the hormones from stimulating the cancer cells to grow. Hormonal therapy is mainly used if the cancer is invasive, but may also be given to women who have DCIS. Tamoxifen| and anastrozole (Arimidex®)| are two commonly used hormonal treatments.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It is not used to treat an underlying DCIS, but may be given if the cancer is invasive.
Herceptin| is one of a new group of drugs called monoclonal antibodies.
Some breast cells divide and grow when a protein (human epidermal growth factor) attaches itself to another protein called HER2. Herceptin blocks this process by attaching itself to the HER2 protein so that the epidermal growth factor cannot reach the breast cancer cells.
Herceptin only works in people who have high levels of the HER2 protein| , which you can be tested for. It can be used to treat early breast cancer or breast cancer which has spread (secondary breast cancer| ).
Research into treatments for Paget’s disease of the breast is ongoing. Cancer doctors use clinical trials| to assess new treatments. Before any trial is allowed to take place, an ethics committee must have approved it and agreed that the trial is in the interest of patients.
You may be asked to take part in a clinical trial. Your doctor must discuss the treatment with you so that you have a full understanding of the trial and what it involves. You may decide not to take part, or withdraw from a trial at any stage. You will then receive the best standard treatment available.
You may have many different emotions| , including anger, resentment, guilt, anxiety and fear. These are all normal reactions, and are part of the process many people go through in trying to come to terms with their illness. You may find it helpful to talk things over with your doctor or nurse, or with one of our cancer support specialists. Close friends and family members can also offer support.
This section has been compiled using information from a number of reliable sources, including:
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